{"id":23934,"date":"2025-09-26T07:58:38","date_gmt":"2025-09-26T07:58:38","guid":{"rendered":"https:\/\/naijaglobalnews.org\/?p=23934"},"modified":"2025-09-26T07:58:38","modified_gmt":"2025-09-26T07:58:38","slug":"friday-briefing-the-huntingtons-treatment-is-a-ray-of-light-for-victims-of-a-brutal-disease-huntingtons-disease","status":"publish","type":"post","link":"https:\/\/naijaglobalnews.org\/?p=23934","title":{"rendered":"Friday briefing: The Huntington\u2019s treatment is a ray of light for victims of a brutal disease | Huntington&#8217;s disease"},"content":{"rendered":"<p>\n<\/p>\n<p class=\"dcr-130mj7b\">Good morning. Few medical diagnoses are as brutal and devastating as Huntington\u2019s disease.<\/p>\n<p class=\"dcr-130mj7b\">For decades, those who inherited this cruel condition faced only despair. There was no cure. Symptoms began with mood swings and depression, then progressed to a loss of movement, followed by dementia, paralysis and, ultimately, death. Some patients died within a decade of diagnosis.<\/p>\n<p class=\"dcr-130mj7b\">Finally, a ray of light has burst through. For the first time, Huntington\u2019s disease has been treated successfully through gene therapy, slowing the progress of the disease by 75% in patients after three years in a groundbreaking trial.<\/p>\n<p class=\"dcr-130mj7b\">It is one of several extraordinary medical advances announced in recent months, alongside new possible treatments and diagnostic tests for Alzheimer\u2019s, heart disease and stroke.<\/p>\n<p class=\"dcr-130mj7b\">For today, I spoke with Hannah Devlin, the Guardian\u2019s science correspondent, about the exciting research behind this desperately needed good news, and why cuts to scientific research could put future progress at risk. That\u2019s after the headlines.<\/p>\n<h2 id=\"five-big-stories\" class=\"dcr-12ibh7f\">Five big stories<\/h2>\n<ol>\n<li class=\"dcr-130mj7b\">\n<p class=\"dcr-130mj7b\"><em><strong>France<\/strong><\/em> | The former French president Nicolas Sarkozy has been found guilty of criminal conspiracy, and given a five-year prison sentence.<\/p>\n<\/li>\n<li class=\"dcr-130mj7b\">\n<p class=\"dcr-130mj7b\"><em><strong>UK politics<\/strong><\/em> | All working adults will need digital ID cards under plans to be announced by Keir Starmer, in a move that will spark a battle with civil liberties campaigners.<\/p>\n<\/li>\n<li class=\"dcr-130mj7b\">\n<p class=\"dcr-130mj7b\"><em><strong>Middle East<\/strong><\/em> | The White House is backing a plan that would see Tony Blair head a temporary administration of the Gaza Strip \u2013 initially without the direct involvement of the Palestinian Authority (PA), according to Israeli media reports.<\/p>\n<\/li>\n<li class=\"dcr-130mj7b\">\n<p class=\"dcr-130mj7b\"><em><strong>US news<\/strong><\/em> | James Comey, the former FBI director and one of Donald Trump\u2019s most frequent targets, was indicted on one count of making a false statement to Congress and one count of obstruction of justice, in the latest move in the president\u2019s retribution campaign against his political adversaries.<\/p>\n<\/li>\n<li class=\"dcr-130mj7b\">\n<p class=\"dcr-130mj7b\"><em><strong>UK news<\/strong><\/em> | The crown court backlog in England and Wales has hit a new record of almost 80,000 cases, while wait times for trial dates have reached up to four years.<\/p>\n<\/li>\n<\/ol>\n<h2 id=\"in-depth-symptoms-in-the-prime-of-your-life\" class=\"dcr-12ibh7f\">In depth: \u2018Symptoms in the prime of your life\u2019<\/h2>\n<p><span class=\"dcr-1inf02i\"><\/span><span class=\"dcr-1qvd3m6\">Cells in the brain of a person with Huntington\u2019s disease. <\/span> Photograph: AP<\/p>\n<p class=\"dcr-130mj7b\">Huntington\u2019s disease is a genetic disease that runs through families. It is caused by a single mutation of a gene that makes a protein called huntingtin.<\/p>\n<p class=\"dcr-130mj7b\">Huntingtin is an essential protein in the brain, but in people who have the mutation, the brain makes a toxic version of it that kills brain cells, Hannah Devlin told me. Between 6,000 and 10,000 people in the UK have the diseases, and another 20,000 are carriers of the faulty gene, which means they are likely to develop it.<\/p>\n<p class=\"dcr-130mj7b\">\u201cIf you\u2019ve got the gene, you tend to get your first symptoms in the prime of your life \u2013 in your 30s and 40s. Then you\u2019ll undergo a mental decline, which is a form of dementia, as well as motor issues; including jerky movements, losing your balance and gradually becoming paralysed,\u201d Hannah said. \u201cIt\u2019s a truly devastating condition, and one of the really awful things about it is if you\u2019re a gene carrier, your children will have a 50% chance of also having the gene.\u201d<\/p>\n<p class=\"dcr-130mj7b\">Over the last 10 years, Hannah has reported on notable progress in the scientific community\u2019s understanding of how the mutant gene causes the disease.<\/p>\n<p class=\"dcr-130mj7b\">She said: \u201cThere has been growing hope that a treatment was on the horizon, but practically, there hasn\u2019t been anything for patients. Until now.\u201d<\/p>\n<p class=\"dcr-130mj7b\"><strong>A one-off injection<\/strong><\/p>\n<p class=\"dcr-130mj7b\">The successful trial used gene therapy to silence the mutant gene so the brain stops producing the toxic protein.<\/p>\n<p class=\"dcr-130mj7b\">The challenge for researchers was overcoming the blood-brain barrier. \u201cWhile drugs can flow around the body, the brain has a special barrier that stops that from happening. So getting any treatment into the brain is itself a massive hurdle,\u201d Hannah said.<\/p>\n<p class=\"dcr-130mj7b\">The breakthrough treatment worked by delivering a small piece of genetic material attached to a harmless virus. Scientists infused the virus directly into the brain. Once inside, the virus entered neurons and delivered the genetic material that silences the mutant gene, stopping it from producing the toxic protein.<\/p>\n<p class=\"dcr-130mj7b\">The procedure is incredibly complex and delicate, Hannah said. \u201cYou have to infuse this virus into the brain very slowly, over 12 to 20 hours, through microcatheters put into the two brain areas that are most affected by Huntington\u2019s.\u201d<\/p>\n<p class=\"dcr-130mj7b\">She added that this gene therapy unit is remarkable because it is effectively a one-off injection. \u201cSo they had the surgery, had this treatment delivered once, and then they were followed up for three years to see whether it altered the progress of the disease.\u201d<\/p>\n<p class=\"dcr-130mj7b\">The research shows that the gene therapy slowed the progress of the disease by 75% in patients after three years.<\/p>\n<p class=\"dcr-130mj7b\"><strong>Ripple of <\/strong><strong>activity<\/strong><\/p>\n<p class=\"dcr-130mj7b\">While political leaders appear intent on delivering doom and gloom, the scientific community seems determined to do the opposite.<\/p>\n<p class=\"dcr-130mj7b\">The breakthrough treatment for Huntington\u2019s disease is the latest in a string of remarkable advances, including promising new therapies and diagnostic testing for Alzheimer\u2019s, heart disease and stroke.<\/p>\n<p class=\"dcr-130mj7b\">\u201cWe have seen some really exciting developments in Alzheimer\u2019s, including the approval of a couple of exciting drugs that could slow progression of the disease. They\u2019re not currently funded by the NHS, but it\u2019s been seen as a big development,\u201d Hannah said.<\/p>\n<p class=\"dcr-130mj7b\">She also pointed to notable breakthroughs in how Alzheimer\u2019s is diagnosed, including a \u00a3100 blood test and a three-minute brain scan, which medics hope could transform diagnosis of the devastating condition. These diagnostics could be critical to getting a much faster diagnosis, which is crucial to treating the disease.<\/p>\n<p class=\"dcr-130mj7b\">\u201cOften when one breakthrough happens, it prompts this ripple of activity and things can suddenly move quite fast in an area where it feels like there was very little progress for years,\u201d Hannah told me.<\/p>\n<p class=\"dcr-130mj7b\">The other big developments recently have been in treatments for heart disease and stroke. Researchers found that a blood-thinning drug could be more effective at preventing heart attacks and stroke than aspirin, which has been the standard for decades.<\/p>\n<p class=\"dcr-130mj7b\">\u201cA lot of this is off the back of big investments into health research,\u201d Hannah said, but that now appears under threat in some corners of the globe.<\/p>\n<p class=\"dcr-130mj7b\"><strong>A bright future, dimmed<\/strong><\/p>\n<p class=\"dcr-130mj7b\">The breakthrough in treating Huntington\u2019s disease would not have happened without public funding.<\/p>\n<p class=\"dcr-130mj7b\">\u201cThis is work that has been going on for more than a decade at UCL [University College London]. This is an absolutely world-leading team that has benefited from public funding into how to study this awful disease. It\u2019s now being fast-tracked in the United States because of this incredible reputation that the FDA [Food and Drug Administration] has with speed and their links with the research community,\u201d Hannah said.<\/p>\n<p class=\"dcr-130mj7b\">But that is now under threat. There have already been major staff layoffs at the FDA, as well as a proposed cut of more than 40% to the National Institutes of Health budget amid what has been called an \u201cassault on science\u201d by the Trump administration. And as I explored in my previous first edition on the impact of US cuts on vaccine research, these decisions will be felt across the world.<\/p>\n<p class=\"dcr-130mj7b\">\u201cIf you fast forward 10 years, some of the research that\u2019s been cut will translate into fewer treatments that could help with some of these really awful diseases. In some cases, researchers are just on the cusp of being able to treat some really devastating diseases so it does feel depressing that these cuts are happening in parallel,\u201d Hannah said.<\/p>\n<p class=\"dcr-130mj7b\">Time will tell whether other countries or global institutions step in to fill the gap left by the US. Until then, I\u2019ll be here to deliver these happy First Editions, only tempered by a bit of caution at the end.<\/p>\n<p>skip past newsletter promotion<\/p>\n<p class=\"dcr-1xjndtj\">Our morning email breaks down the key stories of the day, telling you what\u2019s happening and why it matters<\/p>\n<p><span class=\"dcr-1eusqlu\"><strong>Privacy Notice: <\/strong>Newsletters may contain information about charities, online ads, and content funded by outside parties. If you do not have an account, we will create a guest account for you on theguardian.com to send you this newsletter. You can complete full registration at any time. For more information about how we use your data see our Privacy Policy. We use Google reCaptcha to protect our website and the Google Privacy Policy and Terms of Service apply.<\/span><\/p>\n<p id=\"EmailSignup-skip-link-38\" tabindex=\"0\" aria-label=\"after newsletter promotion\" role=\"note\" class=\"dcr-jzxpee\">after newsletter promotion<\/p>\n<h2 id=\"what-else-weve-been-reading\" class=\"dcr-12ibh7f\">What else we\u2019ve been reading<\/h2>\n<p><span class=\"dcr-1inf02i\"><\/span><span class=\"dcr-1qvd3m6\">A Your Party event in Brixton, south London, earlier this month. <\/span> Photograph: Alishia Abodunde\/Getty Images<\/p>\n<ul class=\"dcr-130mj7b\">\n<li class=\"dcr-130mj7b\">\n<p class=\"dcr-130mj7b\">I was depressed by how quickly Jeremy Corbyn and Zarah Sultana\u2019s <strong>Your Party seemingly fell apart.<\/strong> But this column by Andy Beckett has convinced me to hold my horses before being sure it\u2019s all over for them. <em><strong>Poppy<\/strong><\/em><\/p>\n<\/li>\n<li class=\"dcr-130mj7b\">\n<p class=\"dcr-130mj7b\">A fleet of <strong>civilian vessels is sailing to Gaza<\/strong>. It is believed to be the largest convoy in history to traverse the Mediterranean Sea, with a mission to feed starving Palestinians. David Adler, who is on the boat, explains why he is taking part. <em><strong>Aamna<\/strong><\/em><\/p>\n<\/li>\n<li class=\"dcr-130mj7b\">\n<p class=\"dcr-130mj7b\">I\u2019m not an opera person, but I loved the images in this photo essay by one of the Guardian greats David Levene and a cast of other amazing photographers, an inside look at the <strong>English National Opera\u2019s season-opener. <\/strong><em><strong>Poppy<\/strong><\/em><\/p>\n<\/li>\n<li class=\"dcr-130mj7b\">\n<p class=\"dcr-130mj7b\">I am so buzzed to see the <strong>England <\/strong><strong>Women\u2019s <\/strong><strong>Rugby<\/strong> team in the World Cup final. They are sticking with the same match-day squad that defeated France to take on Canada, with captain Zoe Aldcroft insisting \u201cnow is our time\u201d. Let\u2019s go! <em><strong>Aamna<\/strong><\/em><\/p>\n<\/li>\n<li class=\"dcr-130mj7b\">\n<p class=\"dcr-130mj7b\">The <strong>two-child-benefit cap<\/strong> has become a bit of a political football in recent years, so I felt this video report, showing its real-life impact \u2013 devastating families and forcing them into humiliating compromises \u2013 was much needed. <em><strong>Poppy<\/strong><\/em><\/p>\n<\/li>\n<\/ul>\n<h2 id=\"sport\" class=\"dcr-12ibh7f\">Sport<\/h2>\n<p><span class=\"dcr-1inf02i\"><\/span><span class=\"dcr-1qvd3m6\">John McGinn of Aston Villa celebrates.<\/span> Photograph: Dan Mullan\/Getty Images<\/p>\n<p class=\"dcr-130mj7b\"><em><strong>Fotball <\/strong><\/em>| John McGinn\u2019s first-half strike earned Aston Villa their first win of the season with a 1-0 victory over Bologna in their Europa League opener at Villa Park. Former Celtic striker Oh Hyeon-gyu scored after missing an earlier penalty as Genk beat a 10-man Rangers 1-0 in their Europa League opener at Ibrox.<\/p>\n<p class=\"dcr-130mj7b\"><em><strong>Football <\/strong><\/em>| William Saliba, the 24-year-old centre-back who has been a mainstay of Mikel Arteta\u2019s Arsenal since the 2022-23 season, has agreed a new five-year contract with the team, despite Real Madrid interest<\/p>\n<p class=\"dcr-130mj7b\"><em><strong>Golf <\/strong><\/em>| Club-tangling rough, narrow fairways and a tough workout lie in wait for the USA and Europe in the 45th Ryder Cup, as shown in our hole-by-hole course guide.<\/p>\n<h2 id=\"the-front-pages\" class=\"dcr-12ibh7f\">The front pages<\/h2>\n<p><span class=\"dcr-1inf02i\"><\/span> Photograph: Guardian<\/p>\n<p class=\"dcr-130mj7b\">The <strong>Guardian<\/strong> leads with \u201cMicrosoft blocks Israel\u2019s use of its technology for mass surveillance\u201d. The <strong>Telegraph<\/strong> has \u201cStarmer: Labour got it wrong on migration\u201d. The <strong>i<\/strong> says \u201c\u2018BritCard\u2019 digital ID will be made law for all adults in bid to tackle small boats\u201d. The <strong>Times<\/strong> reports \u201cDigital ID card scheme will confirm right to work\u201d. The <strong>Mirror<\/strong> goes with \u201cPlaying the BritCard\u201d, while the <strong>Mail<\/strong> has \u201cShow us your (digital) papers\u201d. Finally the <strong>Financial Times<\/strong> leads with \u201cBurnham borrowing spree would rock gilts and hurt sterling, investors warn\u201d.<\/p>\n<h2 id=\"something-for-the-weekend\" class=\"dcr-12ibh7f\">Something for the weekend<\/h2>\n<p class=\"dcr-130mj7b\"><em>Our critics\u2019 roundup of the best things to watch, read, play and listen to right now<\/em><\/p>\n<p><span class=\"dcr-1inf02i\"><\/span><span class=\"dcr-1qvd3m6\">An arch schemer whose currency is violence \u2026 James Norton in House of Guinness<\/span> Photograph: Ben Blackall\/Netflix\/PA<\/p>\n<p class=\"dcr-130mj7b\"><strong>TV<\/strong><br \/><strong>House of Guinness<\/strong> <strong>| \u2605\u2605\u2605\u2605\u2605<br \/><\/strong>The family here is not a crime family: we are in Dublin in 1868, where Guinness is so ubiquitous that the unimaginably wealthy Guinness family run the city. But managing the factory that dominates the landscape is the fearsome Sean Rafferty (James Norton), an arch schemer whose currency is violence. He introduces himself by issuing a rallying call to the company workers, exhorting them to crush an anti-Guinness street protest then leading the way himself, gleefully swinging a hunk of hard factory iron. <em><strong>Jack Seale<\/strong><\/em><\/p>\n<p class=\"dcr-130mj7b\"><strong>Music<br \/><\/strong><strong>Olivia Dean: The Art of Loving <\/strong><strong>| \u2605\u2605\u2605\u2605\u2606<br \/><\/strong>Dean\u2019s 2023 debut album, Messy, attracted respectful but mixed reviews and did respectable, rather than remarkable, business. While you wouldn\u2019t describe The Art of Loving as a complete reinvention, it certainly constitutes a noticeable rethink. It expunges most of the cliches of Dean\u2019s debut \u2013 or rather quarantines them on a track called Close Up \u2013 and instead looks for inspiration to music that emanated from recording studios in 70s LA. <em><strong>Alexis Petridis<\/strong><\/em><\/p>\n<p class=\"dcr-130mj7b\"><strong>Film<br \/><\/strong><strong>Sane Inside Insanity: The Phenomenon of Rocky Horror | <\/strong><strong>\u2605\u2605\u2605\u2606\u2606<br \/><\/strong>Andreas Zerr\u2019s unofficial documentary charts the 50-year cult of Richard O\u2019Brien\u2019s beloved classic, from its chaotic early days upstairs at the Royal Court theatre. With archival footage, and cast, fans and producers among the talking heads, it shows how a \u201cshitty fucking movie\u201d became an accidental phenomenon that imitators could never match. <em><strong>Catherine Bray<\/strong><\/em><\/p>\n<p class=\"dcr-130mj7b\"><strong>Theatre<br \/><\/strong><strong>Measure for Measure (Royal Shakespeare theatre, Stratford-upon-Avo<\/strong><strong>n) | \u2605\u2605\u2605\u2605\u2605<br \/><\/strong>Emily Burns\u2019s razor-sharp revival cuts away the comic subplots to deliver a tense, streamlined account of Shakespeare\u2019s play about sexual hypocrisy and power. With slick modern dress, a glass-and-chrome set evoking a political battlefield, and a montage of real-world scandals, the parallels with today are stark. Tom Mothersdale\u2019s Angelo is both appalling and human, while Isis Hainsworth\u2019s Isabella grows from wide-eyed novice to a mighty force. <em><strong>Arifa Akbar<\/strong><\/em><\/p>\n<h2 id=\"today-in-focus\" class=\"dcr-12ibh7f\">Today in Focus<\/h2>\n<p><span class=\"dcr-1inf02i\"><\/span><span class=\"dcr-1qvd3m6\">Detainees are transported at a GEO Group ICE Staging Facility at England Airpark in Alexandria, Louisiana.<\/span> Photograph: Kathleen Flynn\/The Guardian<\/p>\n<p class=\"dcr-130mj7b\"><strong>\u2018Like Amazon Prime but with human beings\u2019: inside Trump\u2019s deportation machine<\/strong><\/p>\n<p class=\"dcr-130mj7b\">Data leak gives Guardian US investigations team an unprecedented look into Trump\u2019s deportation regime \u2013 and how people are seemingly being \u2018disappeared\u2019. Oliver Laughland and Maanvi Singh report.<\/p>\n<h2 id=\"cartoon-of-the-day-ben-jennings\" class=\"dcr-12ibh7f\">Cartoon of the day | Ben Jennings<\/h2>\n<p><span class=\"dcr-1inf02i\"><\/span> Illustration: Ben Jennings\/The Guardian<\/p>\n<h2 id=\"the-upside\" class=\"dcr-12ibh7f\">The Upside<\/h2>\n<p class=\"dcr-130mj7b\"><em>A bit of good news to remind you that the world\u2019s not all bad<\/em><\/p>\n<p><span class=\"dcr-1inf02i\"><\/span><span class=\"dcr-1qvd3m6\">The Argyle Street ash is the only tree on the street.<\/span> Photograph: Douglas Crawford\/Woodland Trust\/PA<\/p>\n<p class=\"dcr-130mj7b\">A much-loved ash tree on Argyle Street in Glasgow has won the tree of the year competition. The towering Victorian-era tree beat the \u201cKing of Limbs\u201d oak in Wiltshire and other majestic specimens in a contest organised by the Woodland Trust. The ash stands 75ft (23 metres) tall on one of the Glasgow \u2019s busiest roads and has survived war, redevelopment and even ash dieback disease. Locals take pride in its elegance \u2013 it has been featured in a book and is celebrated in a nearby pub. This urban survivor will represent the UK in the European contest next spring.<\/p>\n<p class=\"dcr-130mj7b\"><strong>Sign up here for a weekly roundup of The Upside, sent to you every Sunday<\/strong><\/p>\n<h2 id=\"bored-at-work\" class=\"dcr-12ibh7f\">Bored at work?<\/h2>\n<p class=\"dcr-130mj7b\">And finally, the Guardian\u2019s puzzles are here to keep you entertained throughout the day. Until Monday.<\/p>\n","protected":false},"excerpt":{"rendered":"<p>Good morning. Few medical diagnoses are as brutal and devastating as Huntington\u2019s disease. For decades, those who inherited this cruel condition faced only despair. There was no cure. Symptoms began with mood swings and depression, then progressed to a loss of movement, followed by dementia, paralysis and, ultimately, death. Some patients died within a decade<\/p>\n","protected":false},"author":1,"featured_media":23935,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[55],"tags":[1043,2007,4391,1042,14329,2725,5373,2384,2933],"class_list":{"0":"post-23934","1":"post","2":"type-post","3":"status-publish","4":"format-standard","5":"has-post-thumbnail","7":"category-social-issues","8":"tag-briefing","9":"tag-brutal","10":"tag-disease","11":"tag-friday","12":"tag-huntingtons","13":"tag-light","14":"tag-ray","15":"tag-treatment","16":"tag-victims"},"_links":{"self":[{"href":"https:\/\/naijaglobalnews.org\/index.php?rest_route=\/wp\/v2\/posts\/23934","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/naijaglobalnews.org\/index.php?rest_route=\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/naijaglobalnews.org\/index.php?rest_route=\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/naijaglobalnews.org\/index.php?rest_route=\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/naijaglobalnews.org\/index.php?rest_route=%2Fwp%2Fv2%2Fcomments&post=23934"}],"version-history":[{"count":0,"href":"https:\/\/naijaglobalnews.org\/index.php?rest_route=\/wp\/v2\/posts\/23934\/revisions"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/naijaglobalnews.org\/index.php?rest_route=\/wp\/v2\/media\/23935"}],"wp:attachment":[{"href":"https:\/\/naijaglobalnews.org\/index.php?rest_route=%2Fwp%2Fv2%2Fmedia&parent=23934"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/naijaglobalnews.org\/index.php?rest_route=%2Fwp%2Fv2%2Fcategories&post=23934"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/naijaglobalnews.org\/index.php?rest_route=%2Fwp%2Fv2%2Ftags&post=23934"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}